A 22-year-old man with a history of asthma presents to his primary care physician for a checkup. Since his last visit, he has experienced a cough, difficulty breathing, and weight loss. He drinks two to three glasses of wine per week, smokes one to two cigarettes per week, and denies any drug use. Auscultation reveals wheezing, and pulmonary function tests show an increased total lung capacity. Echocardiography is unrevealing. Serum analysis demonstrates elevated liver transaminases.Which of the following is the most likely diagnosis?

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This patient has alpha-1 antitrypsin deficiency, an inherited disorder that affects the lungs and liver.Alpha-1 antitrypsin is a protease inhibitor of elastase. In the lungs, alpha-1 antitrypsin deficiency causes COPD (emphysema and bronchiectasis) due to an imbalance between neutrophil elastase, which destroys elastase, and alpha-1 antitrypsin. This results in uninhibited elastase and decreased elastic tissue. In the liver, unsecreted variant alpha-1 antitrypsin proteins aggregate and polymerize within the endoplasmic reticulum of hepatocytes, causing cirrhosis.Alpha-1 antitrypsin deficiency is an autosomal codominant disorder. Heterozygous individuals are generally asymptomatic but have an increased risk of COPD, are encouraged not to smoke, and often have decreased levels of alpha-1 antitrypsin in the blood.Clinical manifestations of alpha-1 antitrypsin deficiency involve the lung (early-onset panacinar emphysema and bronchiectasis) and liver (cirrhosis).Pulmonary function testing demonstrates obstruction of airflow with increased total lung capacity and decreased forced expiratory volume in one second to forced vital capacity ratio. Chest X-ray reveals bullous changes that are more prominent at the lung bases. Alpha-1 antitrypsin deficiency is diagnosed with serum testing showing a level below 11 micromol/L. Elevated liver enzymes are also commonly seen.Treatment of alpha-1 antitrypsin deficiency involves encouraging patients to avoid tobacco and passive cigarette smoke exposure.Pulmonary function tests are used to monitor lung functions.Pulmonary rehabilitation, nutritional support, supplemental oxygen, preventive vaccinations, and prompt treatment of respiratory infections are all important for these patients. Some patients receive intravenous augmentation with pooled human alpha-1 antitrypsin. Lung and liver transplantation are needed when severe emphysema and end-stage liver disease develop.

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